Blog

Rare or uncommon cases.... sickle cell anaemia

by Work the World

Clinically, one of the main benefits of taking a placement in the developing world is gaining firsthand experience treating diseases that have largely been eradicated from the Western world. You may think that if they don't occur then why do you need to learn about them, but with factors such as climate change, drug-resistant pathogens, and even entirely new diseases, infectious disease spread and containment is a major public health issue worldwide. These eradicated diseases could come back and learning about them firsthand is crucial in your understanding of disease progression and identification of symptoms. There is a fascinating article on the University of Phoenix's website, entitled "Everything old is new again: The re-emergence of eradicated disease" - if that doesn't make you want to find out more, then what kind of healthcare professional are you?!

We are going to focus on some of the rare diseases that you are likely to come across on your elective.... first up sickle cell anaemia.

Disease profile

According to the Sickle Cell Society, the disorder affects the red blood cells which contain a special protein called haemoglobin (Hb for short). The function of haemoglobin is to carry oxygen from the lungs to all parts of the body.People with Sickle Cell Anaemia have Sickle haemoglobin (HbS) which is different from the normal haemoglobin (HbA). When sickle haemoglobin gives up its oxygen to the tissues, it sticks together to form long rods inside the red blood cells making these cells rigid and sickle-shaped. Normal red blood cells can bend and flex easily.

Because of their shape, sickled red blood cells can't squeeze through small blood vessels as easily as the almost donut-shaped normal cells. This can lead to these small blood vessels getting blocked - sickle crisis - which then stops the oxygen from getting through to where it is needed. This in turn can lead to severe pain and damage to organs.

Who does it affect?

The different traits are found mainly in people whose families come from Africa, the Caribbean, the Eastern Mediterranean, Middle East and Asia.

Western World vs Developing Countries

Sickle cell anaemia affects millions of people worldwide, but more than 80 per cent of cases are in Africa where 200,000 children are born with the disease every year.

It is estimated there are over 6,000 adults and children with SCD in Britain at present. Compare this to Tanzania, where 8000 to 11000 new babies are born annually with the disease and you can see the difference. Part of the problem is a lack of knowledge - although childhood mortality rates for SCD are higher than the estimate for HIV, the latter gets far more attention globally. A conference in Dar es Salaam is taking place at the moment to discuss what measures can be taken to prevent further spread and heighten awareness. Dr Soka, Director of Sickle Cell Patients' Services, said:

"Most children with sickle cell die at the age of two due to lack of knowledge about the disease," he said.Sickle cell disease is the most common genetic cause of childhood morbidity. The most severe form is homozygous pattern, known as sickle cell anemia (SCA)".

Tanzania currently ranks fourth in the world for people suffering with the disease, so it's key that researchers and scientists find better ways of managing and improving services related to treatment of the disease.

What experience have our students gained?

"Most mornings began with a ward round led by one of the senior doctors. I learned about many diseases which are just not seen in the UK. Malaria, typhoid and sickle cell crises were very common".James Andrade, Medicine in Ghana, University of Edinburgh

"I spent the first week in general paediatrics, and saw many cases of common childhood illnesses in Tanzania such as malaria, sickle cell disease and malnutrition. Because these conditions are not common in the UK, I had only ever encountered them in textbooks before, so it was useful (though also saddening in many cases) for me to be able to see these conditions for real".Hannah Townsend, Medicine in Dar es Salaam, Hull York Medical School

"I was eager to see around the hospital, I asked if I could organise to visit different wards and clinics. I got the opportunity to visit intensive care ward, psychiatric ward, paediatric ward, malnutrition and sickle cell clinic".Briege McGovern, Nursing in Dar es Salaam, Glasgow Caledonian University